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J Neurol Neurosurg Psychiatry

What is the optimal dose of botulinum toxin A in the treatment of cervical dystonia? Results of a double blind, placebo controlled, dose ranging study using Dysport. German Dystonia Study Group.

Poewe W. Deuschl G. Nebe A. Feifel E. Wissel J. Benecke R. Kessler KR. Ceballos-Baumann AO. Ohly A. Oertel W. Kunig G.
Department of Neurology, University of Berlin, Germany.
OBJECTIVES: Botulinum toxin injections have become a first line therapeutic approach in cervical dystonia. Nevertheless, published dosing schedules, responder rates, and frequency of adverse events vary widely. The present prospective multicentre placebo controlled double blind dose ranging study was performed in a homogenous group of previously untreated patients with rotational torticollis to obtain objective data on dose-response relations. METHODS: Seventy five patients were randomly assigned to receive treatment with placebo or total doses of 250, 500, and 1000 Dysport units divided between one splenius capitis (0, 175, 350, 700 units) and the contralateral sternocleidomastoid (0, 75, 150, 300 units) muscle. Assessments were obtained at baseline and weeks 2, 4, and 8 after treatment and comprised a modified Tsui scale, a four point pain scale, a checklist of adverse events, global assessment of improvement, and a global rating taking into account efficacy and adverse events. At week 8 the need for retreatment was assessed and then the code was unblinded. For those still responding, there was an open follow up until retreatment to assess the duration of effect. RESULTS: Seventy nine per cent reported subjective improvement at one or more follow up visits. Decreases in the modified Tsui score were significant at week 4 for the 500 and 1000 unit groups versus placebo (p

Faecal incontinence in myotonic dystrophy.

Abercrombie JF. Rogers J. Swash M.
Surgical Unit, The Royal London Hospital, UK.
Two siblings with myotonic dystrophy presented for treatment of faecal incontinence. The pathophysiology of this functional disorder is described with the results of anorectal manometry, EMG, and biopsy of smooth and striated muscle of the anorectal sphincters. Both medical and surgical management of the incontinence was unsatisfactory in the long term. Involvement of gastrointestinal musculature is a characteristic feature the disease.

Cough responsiveness in neurogenic dysphagia.

Year 1998
Smith PE. Wiles CM.
Department of Medicine (Neurology), University of Wales College of Medicine, Cardiff, UK.
OBJECTIVES: In neurogenic dysphagia a good cough is important for airway protection. If triggering of cough, or its effectiveness, is impaired this might result in an increased aspiration risk. Capsaicin, an agent which induces cough through sensory nerve stimulation, was used to test cough sensitivity in groups of patients with and without neurogenic dysphagia. METHODS: On the basis of swallowing speed (ml/s) in a validated water test 28 alert neurological inpatients (16 women, aged 22-71 years) were classified into 13 with abnormal and 15 with normal swallowing (median swallowing speed 23% and 99%, median volume/swallow 43% and 106% of that predicted for age and sex respectively: p

Common variable immunodeficiency leading to spinal subacute combined degeneration monitored by MRI.

Year 1998
Yousry TA. Strupp M. Bruning R.
Department of Neuroradiology, Klinikum Grosshadern, Ludwig-Maximilian University, Munich, Germany. Yousry@ikra.med.uni-muenchen.de
A patient is reported on with a common variable immunodeficiency syndrome (CVID), in whom chronic gastritis with antibodies against parietal cells and a cytomegalovirus associated enteritis led to vitamin B12 deficiency with consecutive subacute combined degeneration of the spinal cord. The resulting medullary changes, most probably representing demyelination, were visualised with MRI. The effects of treatment were also monitored over three years by MRI and clinical examination. The resolution of the MRI changes correlated with clinical improvement of the patient's signs. In the medical literature only five cases of vitamin B12 related spinal cord changes have been identified by MRI; none was caused by a CVID syndrome.

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